摘要： Background: Carotid endarterectomy (CEA) prevents the occurrence of stroke in the future, although its efficacy depends on the detection and control of high perioperative risk factors. We aimed to analyze the association between preoperative neurological deficit and the 30-day risk of major adverse cardiovascular events (MACEs) in CEA with selective shunting for symptomatic carotid stenosis. Methods: We assessed 653 patients who underwent CEA with selective shunting for symptomatic carotid stenosis between August 2011 and August 2019. The primary outcomes of the study were the occurrence of MACEs, defined as stroke (ischemic stroke or cerebral hemorrhage), all-cause mortality, and myocardial infarction during the perioperative period after CEA. Baseline patient characteristics were analyzed to identify factors associated with perioperative (<30 days) MACEs. Multivariable logistic regression models were used to estimate the association between preoperative modified Rankin Scale (mRS) and the 30-day risk of MACEs. Interaction and stratified analyses were conducted according to age, drinking, history of hypertension and coronary artery disease, and surgical side. Results: The mean age of patients was 68.7 +/- 9.1 years, and 86.4% were men. The 30-day MACEs rate was 2.5%. In univariate logistic regression, histories of coronary artery disease (odds ratio (OR), 2.57 [95% confidence interval (CI), 1.04-6.34]), a severe contralateral carotid stenosis or occlusion (OR, 4.52 [95% CI, 1.84-11.11]), and a poor neurological deficit (mRS >= 3 versus mRS < 3: OR, 3.78 [95% CI, 1.21-11.82]) were associated with higher primary outcome rates. A history of hypertension did not increase the risk of MACEs (OR, 0.37 [95% CI, 0.15- 0.89]). In the multivariable regression analysis, poor neurological deficit was associated with a higher risk of the MACEs within 30 days (mRS >= 3 versus mRS < 3: 11.1% vs. 2.0%, adjusted OR 5.70 [95% CI, 1.50-21.60]). The interaction analysis revealed no int

关键词： Gaucher disease type 3   Neuronopathic form   Ambroxol   Pharmacological chaperone  

摘要： Gaucher disease type 3 (GD3) is a severely debilitating disorder characterized by multisystemic manifestations and neurodegeneration. Enzyme replacement therapy alleviates visceral signs and symptoms but has no effect on neurological features. Ambroxol has been suggested as an enzyme enhancement agent. Some studies have confirmed its effectiveness in preventing the progression of neurological manifestations of neuronopathic Gaucher disease. In this study, we report two GD3 siblings in whom ambroxol combined with enzyme replacement therapy was initiated at different stages of the disease. We demonstrate the enzyme enhancement effect of ambroxol on L444P/H225Q;D409H glucocerebrosidase activity through results of fibroblast studies and long-term clinical outcomes of the two patients. The sibling diagnosed at the age of four-and-a-half years with significant neurological involvement manifested relatively rapid improvement on ambroxol treatment, followed by stabilization of further course. The younger sibling, in whom the treatment was started at seven weeks, displayed attention deficit and low average cognitive functioning at the age of seven years, but did not manifest other neurological symptoms. The difference in neurological outcomes indicates that ambroxol delayed or even halted the evolution of neurological manifestations in the younger sibling. This observation suggests that early initiation of ambroxol treatment may arrest neurological involvement in some GD3 patients. (c) 2021 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

关键词： Adult brain   Neurogenesis   Hippocampus   Direct reprogramming   Epigenetics   Injury   Microglia  

摘要： Neural stem cells (NSCs) in the adult hippocampus generate new neurons via a process referred to as neurogenesis, supporting cognitive functions. Since altered neurogenesis has been reportedly associated with several diseases such as epilepsy, the molecular basis of NSC activity is an important focus in the study of neurogenesis. Furthermore, facilitation of neurogenesis in the injured brain would be an ideal approach to replenish lost neurons for damage recovery. However, natural neurogenesis by endogenous NSCs in the adult brain is insufficient for complete recovery after severe injury. Recent advances in understanding forced neurogenesis from brain-resident non-neuronal cells by direct reprogramming and clearing hurdles to achieve it have improved the ability to replace damaged neurons in the brain. In this review, we describe molecular mechanisms underlying natural and forced neurogenesis, and discuss future directions for treatments of diseases in the central nervous system. (c) 2020 Elsevier B.V. and Japan Neuroscience Society. All rights reserved.

关键词： cohort study <   research methods   functional disorders <   psychiatric disorders <   neurological disorders   psychiatric disorders <   neurological disorders  

摘要： Background Functional neurological disorders (FND) represent a significant proportion of presentations to outpatient adult neurology services. There is little information relating to patients presenting to acute inpatient care. Methods We identified patients presenting as acute admissions with FND to Christchurch Hospital, Christchurch, New Zealand, from 2016 to 2018. We analyzed relevant demographic and clinical data from electronic records and measured incidence of presentation to secondary care and healthcare utilization. Results One hundred sixty-two patients presented on 173 occasions with FND, representing 9% of all admissions to the neurology service during the 3-year study period. The mean age was 40 (SD 17) years, 111 (69%) patients were female and the median length of stay was 3 (IQR 2-4) days. A total of 92 computed tomography brain scans, 77 magnetic resonance imaging brain scans and 42 electroencephalograms were carried out. On 22 (13%) occasions, patients were referred for outpatient psychological therapy. In the 3 years prior to each patient's last presentation in the study period, these 162 patients had a total of 671 presentations to the emergency department. Healthcare demand did not decrease after the index admission. The rate of acute inpatient admission for FND was 10 per 100,000 per year for the total Christchurch Hospital catchment, 6/100,000/year in rural areas, and 11/100,000/year in urban areas. Conclusion FND represented almost 1 in 10 acute neurology admissions with significant inpatient healthcare resource utilization.

关键词： Outcomes research   Spinal cord diseases  

摘要： Study design Retrospective multicenter study. Objectives To identify the usefulness of the baseline severity of myelopathy for predicting surgical outcomes for cervical spondylotic myelopathy (CSM). Setting Seventeen institutions in Japan. Methods This study included 675 persons with CSM who underwent posterior decompression. According to baseline severity, the individuals were divided into the mild (Japanese Orthopaedic Association [JOA] score >= 14.5), moderate (JOA score = 10.5-14), and severe (JOA score <= 10) groups. Surgical outcomes and clinical variables were compared between the groups. Logistic regression analysis was used to develop a prediction model for unsatisfactory symptom state (postoperative JOA score <= 14, residual moderate or severe myelopathy). Results The mean (+/- standard deviation) age was 67 +/- 12 years. The participants in the severe group were older than those in the mild group. Postoperative JOA scores were higher in the mild group than in the severe group. According to multivariate logistic regression analysis, the prediction model included preoperative JOA scores (odds ratio [OR] 0.60;95% confidence interval [CI] 0.55-0.67) and age (OR 1.06, 95% CI 1.04-1.08). On the basis of the model, a representative combination of the thresholds to maximize the value of "sensitivity - (1 - specificity)" demonstrated a preoperative JOA score of 11.5 as a predictor of postoperative unsatisfactory symptom state in people around the mean age of the study cohort (67 years). Conclusions The combination of the baseline severity of myelopathy and age can predict postoperative symptom states after posterior decompression surgery for CSM.

关键词： Case report   Supracondylar fracture   Radial nerve   Transection  

摘要： INTRODUCTION AND IMPORTANCE: Traumatic laceration of the radial nerve following supracondylar humerus fracture in the pediatric population is highly uncommon. Transection of the radial nerve is rare, and approximately 12.7% of all nerve injuries are categorized as neurapraxia that will resolve spontaneously. CASE PRESENTATION: We report a rare case of a 9-year-old boy presented with a right wrist drop after fell on an outstretched hand two weeks before. The radiological finding revealed a posteriorly displaced supracondylar humerus fracture. Intraoperatively, we found the radial nerve had lost its continuity and interposed by fibrotic tissue. We resect and do a direct repair of the nerve along with fixation of the fracture. At 4-months postoperative follow-up, the patient could fully extend his right thumb and wrist but retain a tingling sensation in the radial nerve distribution. At 1-year follow up the motor and sensory functions were normal without any complication. CLINICAL DISCUSSION: Transection of radial nerve following the supracondylar fracture represents a rare case. The radial nerve was transected and interposed by scar tissue at the fracture site level, preventing spontaneous nerve regeneration. In a case of nerve transection, surgical repair is indicated. CONCLUSION: In the case of supracondylar humerus fracture with posterior displacement, radial nerve transection could be associated with injuries. Authors favour nerve exploration with fracture stabilization performed as soon as possible when nerve injury is suspected. Appropriately managed, the outcome in the pediatric population is highly favourable. (C) 2021 The Authors. Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd.

关键词： Neurocritical care in pregnancy   Stroke in pregnancy   Seizures in pregnancy   Intracerebral hemorrhage in pregnancy   Cerebral venous sinus thrombosis   Posterior reversible encephalopathy syndrome   Neuroimmune disorders in pregnancy   Brain death in pregnancy  

摘要： This article serves as a comprehensive review of neurologic emergencies in pregnancy. Common initial presentations of neuroemergencies are reviewed with a clinical algorithm. Neurovascular disorders in pregnancy are reviewed, including acute ischemic strokes with data on tPA and thrombectomy; cerebral venous sinus thrombosis diagnosis and management; posterior reversible encephalopathy syndrome management; reversible cerebral vasoconstriction syndrome; and intracerebral hemorrhage. Attention is paid to management of cerebral edema and safety of osmotic therapies. A review of seizures in pregnancy is provided, including new-onset causes and decreased seizure thresholds. For status epilepticus, a low teratogenicity risk medication protocol in pregnancy is provided.

关键词： Neurological outcome   Out of hospital cardiac arrest   Neuropsychology   Hypothermic treatment  

摘要： Background: Patients surviving out-of hospital cardicac arrest, with good neurological outcome according to Cerebral Performance Category, frequently have neuropsychological impairment. We studied whether biomarker data (S-100b and neuron-specific enolase) obtained during the ICU stay predicted cognitive impairment 6 months after resuscitation. Methods: Patients (N = 79) with a CPC-score <= 2 were recruited from two trial sites taking part in the TTH48 trial comparing targeted temperature management (TTM) for 48 h vs. 24 h at 33 +/- 1 degrees C. We assessed patients 6 months after the OHCA. We measured biomarkers S-100b and NSE at arrival and at 24, 48 and 72 h after reaching the target temperature of 33 +/- 1 degrees C. Four cognitive domain z-scores were calculated, and global cognitive impairment was defined as z < -1.67 on at least 3 out of 13 cognitive tests. Nonparametric correlations were used to assess the relationship between cognitive domain and biomarkers. ROC curves were used to assess prediction of cognitive impairment from the biomarkers. Logistic regression was used to investigate whether TTM duration moderated biomarker prediction of cognitive impairment. Results: Cognitive impairment was present in 22% of the patients with memory impairment being the most common. The biomarkers correlated significantly with several cognitive domain scores and NSE at 48 h predicted cognitive impairment with 100% sensitivity and 56% specificity. The predictive properties of NSE at 48 h was unaffected by duration of TTM. Conclusions: Early biomarker prognostication of cognitive impairment is feasible even in OHCA survivors with good neurological outcome as defined by CPC. NSE at 48 h predicted cognitive impairment.

关键词： TBI   RP101075   Neurological deficit   Cognitive dysfunction  

摘要： Background and Purpose: The inflammatory response after traumatic brain injury (TBI) can contribute to secondary brain injury. RP101075, a sphingosine-1-phosphate receptor modulator, can attenuate various inflammatory responses. Here, we hypothesized that consecutive administration of RP101075 over 3 days could broadly suppress the TBI-induced inflammatory response and ameliorate the outcomes of TBI. Methods and Results: Young C57/BL6 mice were subjected to a controlled cortical impact (CCI) model. RP101075-treated mice exhibited significantly reduced scores on the modified neurological severity score (mNSS) test on days 3, 7, 14, and 21 after TBI, in comparison to TBI mice that received the vehicle. RP101075-treated mice had a remarkably decreased percentage of foot faults on the foot fault test on days 7, 14, and 21 after surgery, in comparison to TBI mice that received the vehicle. Using the wet brain weight/dry brain weight method, we found that RP101075 attenuated brain edema at 3 days post-TBI. According to the results of the Morris water maze (MWM), TBI mice treated with RP101075 exhibited reduced latency time and an increased percentage of target quadrant time from day 24 to day 25 after TBI, in comparison to TBI mice that received the vehicle. In addition, flow cytometry and immunohistochemistry showed that RP101075 markedly decreased the number of infiltrated T cells, B cells and NK cells at 3 days after TBI. Analysis of Western blot data showed that RP101075 lowered the expression of proinflammatory factors on day 3 after TBI. Conclusions: Our study demonstrated that consecutive administration of RP101075 over 3 days suppressed the TBI-induced inflammatory response and ameliorated neurological deficits after TBI. Thus, this procedure may be a potential treatment strategy for TBI in the clinical setting.